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溶血尿毒综合征:自然病史及预后特征分析

Hemolytic-uremic syndrome: an analysis of the natural history and prognostic features.

作者信息

Loirat C, Sonsino E, Varga Moreno A, Pillion G, Mercier J C, Beaufils F, Mathieu H

出版信息

Acta Paediatr Scand. 1984 Jul;73(4):505-14. doi: 10.1111/j.1651-2227.1984.tb09962.x.

Abstract

Sixty-seven children with hemolytic-uremic syndrome (HUS) were admitted between 1974 and 1981. Of these, 52 (78%) were aged less than 3 years. All children had acute renal failure and 48 (72%) required peritoneal dialysis. The etiology in twenty cases varied from bacterial and viral infections (7 and 5 cases, respectively) to renal irradiation with chemotherapy (2) and preexisting glomerulopathy (1). 5 (7%) children died during the acute phase of the illness. Long-term follow-up (mean 3 years 3 months) of 56 cases showed that 37 children (60%) had so far experienced no functional sequelae and 8 (13%) only mild sequelae while 3 (5%) were on iterative hemodialysis, 3 had severe chronic renal failure and high blood pressure (HBP) and 5 (8%) had HBP and normal kidney function. While the recovery rate was approximately 60% in all age groups, the mortality rate and serious after-effects were twice as frequent (42%) in children over 3 years of age as in those less than 3. Renal histology (total of 37) showed 12 cases of cortical necrosis, 22 of glomerular thrombotic microangiopathy (TMA) and 3 arterial TMA. Prognosis was poor for all cases of arterial TMA and 58% of those exhibiting cortical necrosis.

摘要

1974年至1981年间,收治了67例溶血尿毒综合征(HUS)患儿。其中,52例(78%)年龄小于3岁。所有患儿均有急性肾衰竭,48例(72%)需要进行腹膜透析。20例患儿的病因各不相同,从细菌和病毒感染(分别为7例和5例)到肾放疗联合化疗(2例)以及既往存在的肾小球病(1例)。5例(7%)患儿在疾病急性期死亡。对56例患儿进行长期随访(平均3年3个月)发现,37例患儿(60%)目前未出现功能后遗症,8例(13%)仅有轻度后遗症,3例(5%)需要反复进行血液透析,3例有严重慢性肾衰竭和高血压(HBP),5例(8%)有HBP但肾功能正常。虽然所有年龄组的恢复率约为60%,但3岁以上儿童的死亡率和严重后遗症发生率是3岁以下儿童的两倍(42%)。肾组织学检查(共37例)显示,12例为皮质坏死,22例为肾小球血栓性微血管病(TMA),3例为动脉TMA。所有动脉TMA病例以及58%表现为皮质坏死的病例预后均较差。

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